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  • 1
    Electronic Resource
    Electronic Resource
    Treatment of children with haemophilia in Europe: a survey of 20 centres in 16 countries (2000)
    Oxford UK : Blackwell Science Ltd
    Haemophilia 6 (2000), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: A survey was made of the current status of treatment of haemophilic boys at 20 centres in 16 European countries and includes approximately 1500 of the estimated 6500 haemophiliacs in the participating countries. Many mild haemophiliacs are not seen, or seen infrequently, at haemophilia centres and this requires study. Nine of 18 centres provide continuous prophylaxis to 80–100% of their patients, five centres provide it to 55–80% and the remaining four centres to 15–40% of the boys. The median dose given was 6240 U kg−1 year−1 (range 3120–7800). Four centres administered only recombinant concentrates to children with severe haemophilia A, while seven centres administered recombinant concentrates to 75–90% and the remaining centres to less than 50% of the boys (two centres 〈 10%). When asked for the choice of concentrate for a newly diagnosed boy with severe haemophilia A, all but one centre preferred recombinant concentrate. Most boys below 6 years received concentrates via a peripheral vein but three centres preferred a central venous line for 80–100% of the boys. Thirteen of 18 centres applied home treatment to 84–100% of the boys and the remaining five centres to 57–77% of the boys.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2516.2000.00427.x
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  • 2
    Electronic Resource
    Electronic Resource
    High-dose intravenous immunoglobulins in the treatment of adolescent and adult HIV-infected hemophiliacs (1994)
    Springer
    Journal of molecular medicine 72 (1994), S. 122-126 
    Details
    ISSN: 1432-1440
    Keywords: Hemophiliacs ; Human immunodeficiency virus infection ; Acquired immunodeficiency syndome ; Intravenous immunoglobulin ; Therapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In children infected with human immunodeficiency virus (HIV) placebo-controlled trials with intravenous immunoglobulins have resulted in a significant reduction in morbidity; however, the results of small trials in adolescents and adults have been inconsistent. In this study 17 HIV-infected hemophiliacs aged 9–30 years were treated with monthly intravenous immunglobulins for an average of 32 months. At the end of the study, 8 years after the HIV infection, three patients (18%) had progressed to the acquired immunodeficiency syndrome(AIDS), and the average decrease in CD4 cells was 81 cells/μl per year. The natural history of HIV infection in hemophiliacs in this age group shows a manifestation rate of AIDS between 11 and 26% 6–8 years after seroconversion and an average yearly decrease in CD4 lymphocytes of 68–110 cells/μl. In conclusion, we observed no difference either in the manifestation rate of AIDS or in prognostic markers in this small cohort of HIV-infected hemophiliacs treated for more than 30% of their latency period with intravenous immunoglobulins compared to the well-documented natural history of HIV-infected hemophiliacs. However, none of the patients developed severe bacterial infections during the study period.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00184588
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  • 3
    Electronic Resource
    Electronic Resource
    Homozygoter Säugling in einer Sippe mit erblichem Protein C-Mangel (1987)
    Springer
    Journal of molecular medicine 65 (1987), S. 576-580 
    Details
    ISSN: 1432-1440
    Keywords: Protein C deficiency ; Thrombophilia ; Microthrombosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We report on a female infant homozygous for protein C deficiency in a Jordanian family with frequent intermarriage. A protein C antigen of 0.6% was determined. The parents first noticed painful nodular indurations in subcutanous tissue as well as blue-red skin coloration at the age of 6 months. The girl repeatedly suffered from micro-thrombotic events in parts of the body with large areas of subcutaneous fat. In contrast, the numerous heterozygous carriers with partial protein C deficiency did not show an increased tendency to thrombosis. From the history an autosomal-recessive inheritance may be inferred. Other authors reporting on homozygous cases also postulate the presence of a recessive gene. It is of interest that the infant described here differs from those in other case reports in the age at manifestation of the disease. The homozygous infant showed the first symptoms as late as the age of 6 months, whereas other case reports describe severe symptoms immediately after birth. All symptoms of disease were treated successfully with prothrombin complex concentrate without additional heparin protection. Microthrombotic events subsided quickly, and a large ulcer in the left flank healed almost completely within 6 days.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01727627
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  • 4
    Electronic Resource
    Electronic Resource
    Obstruktive kongenitale Dakryozystozelen (1999)
    Springer
    Monatsschrift Kinderheilkunde 147 (1999), S. 652-654 
    Details
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Dakryozystozele ; Kongenital ; Atemnot ; Key words Dacryocystocele ; Congenital ; Respiratory distress
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Obstructions of the nasolacrimal duct occur frequently (70%) in term newborns and are symptomatic in 6%. The reason is an incomplete canalization and differentiation of the lacrimal duct during the fetal period. A persisting membran between the duct and the nasal cavity at the level of the valve of Hasner in combination with a proximal valvelike obstruction at the junction of the lacrimal canal and sac may cause a dacryocystocele by fluid accumulation. We describe a term newborn with a bilateral dacryocystocele and respiratory distress. Discussion: The bilateral dacryocystocele can cause respiratory distress in newborns and appears clinically like a choanal stenosis. The surgical removal of the intranasal cysts leads to a prompt healing.
    Notes: Zusammenfassung Tränennasengangsstenosen sind bei Neugeborenen häufig. Sie finden sich bei 70% der Neugeborenen und verursachen bei 6% klinische Symptome. Die Ursache ist in der Regel eine unvollständige Kanalisierung bzw. Ausdifferenzierung des fetalen Tränennasengangs. Dabei kann eine Membran im Bereich der Hasner-Klappe an der Mündungsstelle des Tränennasengangs in den unteren Nasengang persistieren. Zusätzlich kann zu dieser distalen eine proximale Obstruktion mit Ventilmechanismus an der Einmündung der Tränenkanäle in den Tränensack bestehen. Der hierdurch verursachte Sekretstau führt zu einer Dakryozystozele. Wir berichten über ein Neugeborenes mit beidseitiger Dakryozystozele und Atemnot. Diskussion: Eine beidseitige Dakryozystozele kann bereits Neugeborenen die oberen Atemwege obstruieren und klinisch eine Choanalstenose imitieren. Die operative Spaltung der intranasalen Zysten führt zur sofortigen Heilung.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001120050476
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  • 5
    Electronic Resource
    Electronic Resource
    Evaluation of a new protein-C concentrate and comparison of protein-C assays in a child with congenital protein-C deficiency (1992)
    Springer
    Annals of hematology 64 (1992), S. 146-151 
    Details
    ISSN: 1432-0584
    Keywords: Protein-C concentrate ; Protein-C deficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We investigated a new protein-C (PC) concentrate in a child with a type-II homozygous deficiency, concerning tolerance and safety. By means of various functional and antigen assays the in vivo recovery and the half-life were determined. In order to compare the results we reduced the measured values to the average halfrlife of 10.0 ± 0.5 h and to an optimal recovery of 96.6%. Considerable discrepancies observed in the response of functional (clotting) and both the amidolytic and antigen assays are characteristic for type II and anticoagulant treatment. The substituted protein C is activated by the endogenous system. Thus, the efficacy of activation can be determined in deficiency states. The antigen activity of one unit PC concentrate was found to be 120% (or 1.2 U/ml plasma), close to the 100% activity defined for endogenous PC.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01697402
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  • 6
    Electronic Resource
    Electronic Resource
    Prevalence and outcome of intracranial haemorrhage in haemophiliacs – a survey of the paediatric group of the German Society of Thrombosis and Haemostasis (GTH) (1999)
    Springer
    European journal of pediatrics 158 (1999), S. S162 
    Details
    ISSN: 1432-1076
    Keywords: Key words Haemophilia ; Intracranial haemorrhage ; Prevalence ; Neurological outcome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A survey among centres of the paediatric group of the GTH was performed to evaluate the prevalence and outcome of haemophiliacs with intracerebral haemorrhage. A questionnaire sent to the centres covered the following points: number of patients with severe, moderate and mild haemophilia A and B; for each patient with ICH: birth date, age at bleeding, aetiology and neurological sequelae. Overall, 30 ICH in 744 haemophiliacs (4.0%) were reported by 17/40 centres (42.5%). There was no significant difference between the prevalence of patients with haemophilia A and B (3.5% vs. 6.3%) and among the age groups. Bleeding was diagnosed within 1 week of birth in 11/27 patients (41%). For 3 patients, no age-related information was given. The most important factor was trauma (17/30 = 57%), either during birth (9/30 = 30%) or later in life (8/30  = 27%). Seizures were common, occurring in 19/30 patients (63%). As 1 patient died after posttraumatic ICH, the neurological outcome of 29 patients could be evaluated. Psychomotor and statomotor retardation and cerebral palsy were reported in 17/29 (59%), 15/29 (51%) and 13/29 (45%) patients respectively. Only 7/29 (24%) showed no neurological sequelae. Severity of deficits was not correlated with birth date but to age at bleeding. Older children showed a better neurological outcome than neonates. Conclusion The frequency and outcome of ICH in haemophiliacs have not changed in our cohort over the past 20 years. Trauma at birth is an important risk factor for ICH in patients with haemophilia A or B. lntracranial haemorrhages in older children are rare, and a better outcome may be expected.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/PL00014346
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  • 7
    Electronic Resource
    Electronic Resource
    The 677T genotype of the common MTHFR thermolabile variant and fasting homocysteine in childhood venous thrombosis (1999)
    Springer
    European journal of pediatrics 158 (1999), S. S113 
    Details
    ISSN: 1432-1076
    Keywords: Key words MTHFR C677T ; Homocysteine ; F V G1691A ; Prothrombin G20210A ; Childhood venous thrombosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Controlled data on the association of MTHFR genotypes, hyperhomocysteinaemia and their interaction with factor V G1691A with childhood thrombosis are not yet available. Therefore we conducted a case-control study comparing 141 childhood patients with venous thrombosis with 345 healthy controls. The MTHFR C677T genotypes, FV G1691A and prothrombin G20210A were evaluated; in addition, fasting homocysteine concentrations were measured in a subgroup of 60 children and 80 healthy controls. 10.4% of the healthy control population showed the MTHFR TT genotype, 34.2% the CT genotype and 55.4% the CC variant. MTHFR genotypes account for fasting homocysteine concentrations in healthy controls (CC: 5.5 μmol/l (4–7.2); CT: 7 μmol/l (3.9–9.8); TT: 12.1 μmol/l (7.7–13.3)) with an upper age-specific 95th percentile of 8.3 μmol/l. The following frequencies (patients versus controls), odds ratios (OR) and 95% confidence intervals (CI) were found for single defects: MTHFR 677TT genotype (10.6% vs. 10.4%; OR/CI: 1.02/0.54–1.93; P= 0.99) and CT genotype (43.8% vs. 34.2%; OR/CI: 2.12/1.42–3.16; P= 0.0000). A combination of FV G1691A mutation and MTHFR 677CT genotype was found in 9.9% of patients and in 2.9% of the controls (OR/CI: 3.8/1.64–8.75; P= 0.027). Fasting homocysteine median (range) concentrations in the patient group were significantly higher than in the controls (7 μmol/l (3–23) vs. 5.5 μmol/l (3–8.4); P= 0.0004), and homocysteine concentrations 〉8.3 μmol/l were found in 40% of patients vs. 2.5% of the controls (OR/CI: 22/2.64–183; P= 0.0003). Conclusion Data of this childhood case-control study suggest that mildly elevated fasting homocysteine concentrations 〉8.3 μmol/l and the CT genotype of the MTHFR C677T variant are significant risk factors for venous vascular occlusion in children.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/PL00014332
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  • 8
    Electronic Resource
    Electronic Resource
    Factor V G1691A and prothrombin G20210A in childhood spontaneous venous thrombosis – Evidence of an age-dependent thrombotic onset in carriers of factor V G1691A and prothrombin G20210A mutation (1999)
    Springer
    European journal of pediatrics 158 (1999), S. S105 
    Details
    ISSN: 1432-1076
    Keywords: Key words Spontaneous thrombosis ; Paediatrics ; Factor V G1691A ; Prothrombin G20210A ; Age-dependency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Risk factors for venous thrombosis in adults are the prothrombin (PT) G20210A, the factor (F) V G1691A mutations and hereditary deficiencies of protein C, protein S and antithrombin. However, data are limited on the relevance of these risk factors for thrombosis in children and adolescents. We therefore investigated 119 patients aged 0–18 with spontaneous venous thrombosis and controls (n= 100) for the presence of the factor V G1691A mutation and the prothrombin G20210A variant with respect to thrombotic onset and thrombosis location. The following frequencies (patients vs. controls), odds ratios (OR), 95%-confidence intervals (CI) and p-values were found: FV G1691A, 19.3% vs. 5%, OR/CI 4.55/1.66–12.5, p= 0.0038 and prothrombin G20210A, 8.4% vs. 3%, OR/CI 2.96/0.8–11, p= 0.17. A combination of the FV G1691A mutation with the PT G20210A variant was found in 3 children (2.5% of cases) but only once in the controls. With a median (range) age of 2 years (0–17), carriers of the FV mutation were significantly younger compared with patients carrying the PT variant (16 years: 0–18, p 〈 0.001). Vascular accidents in carriers of the FV mutation occurred in deep veins of the leg (n= 11), cerebral veins (n= 4), renal veins (n= 3) and portal veins (n= 2). Patients with the PT mutation showed spontaneous thrombosis in the majority of cases in the deep veins of the leg (n= 5) and in the central nervous system (n= 2). Combined defects were found in a neonate with renal venous thrombosis and in two adolescents with deep vein thrombosis. Conclusion Data presented here suggest that the heterozygous FV mutation is the most commonly found prothrombotic risk factor responsible for spontaneous thrombosis during infancy and early childhood. In contrast, the PT G20210A variant is likely to be more important during puberty and adolescence.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/PL00014335
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  • 9
    Electronic Resource
    Electronic Resource
    Role of genetic prothrombotic risk factors in childhood caval vein thrombosis (1999)
    Springer
    European journal of pediatrics 158 (1999), S. S109 
    Details
    ISSN: 1432-1076
    Keywords: Key words Vena caval thrombosis ; Paediatrics ; Factor V G1691A ; Prothrombin G20210A ; Lipoprotein (a)
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Childhood caval vein thrombosis has a high incidence especially in the first year of life. Besides deficiencies of protein C, protein S, antithrombin and plasminogen, the factor (F) V G1691A mutation, the prothrombin (PT) G20210A variant, the methylenetetrahydro-folate reductase (MTHFR) TT677 genotype, or increased lipoprotein (Lp) (a)〉30 mg/dl have emerged as important prothrombotic risk factors in childhood vascular accidents. 27 consecutive childhood patients with inferior caval vein thrombosis and 100 healthy age-matched controls were investigated for the presence of these prothrombotic risk factors with respect to the first thrombotic onset. In 19 out of 27, patients thrombosis occurred during infancy; the remaining vascular accidents were diagnosed during puberty. In 13 out of the 19 infants, vascular occlusion occurred spontaneously, five times associated with renal venous thrombosis. 68.4% of patients in the first year of life (n=13) showed at least one prothrombotic risk factor. The FV mutation (heterozygous n=4, homozygous n=1), Lp (a)〉30 mg/dl and kringle 4 repeats 〈28 (n=4), MTHFR TT677 with mild hyperhomocysteinaemia (〉95th age-dependent percentile, i.e. 8.5 μmol/l: n=3) and antithrombin deficiency type II (n=1) were diagnosed with an overall odds ratio/95% confidence interval of 9.2/3.1–27.4. In the adolescent group, genetic risk factors were found in 50% of patients investigated (FV mutation (n=1), PT variant (n=3); odds ratio/95% confidence interval: 4.2/0.97–18.6). Conclusion Data presented here suggest that genetic prothrombotic risk factors play an important role in childhood caval vein thrombosis. Remarkably, during puberty and adolescence the predominant defect diagnosed was the PT G20210A variant, whereas the FV G1691A mutation had a higher incidence during infancy.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/PL00014334
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  • 10
    Electronic Resource
    Electronic Resource
    The low molecular weight heparin dalteparin for prophylaxis and therapy of thrombosis in childhood: a report on 48 cases (1999)
    Springer
    European journal of pediatrics 158 (1999), S. S134 
    Details
    ISSN: 1432-1076
    Keywords: Key words LMWH ; Anti-Xa activity ; Veno-occlusive lung disease ; Primary pulmonary hypertension ; Thrombosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We investigated the efficacy, safety and relation of dose to plasma anti-Xa activity of the low molecular weight heparin (LMWH) dalteparin in prophylaxis and therapy of arterial and venous thrombosis in pediatric patients. A total of 48 children were enrolled: 10 received dalteparin for prophylaxis (group I), 8 for reocclusion prophylaxis following successful thrombolysis (group II), 5 following failed thrombolysis (group III) and 23 for primary antithrombotic therapy (group IV). Two children were treated with dalteparin for pulmonary veno-occlusive disease (PVOD) and for primary pulmonary hypertension (PPH), respectively. Outcome: In group I no thrombo-embolic event occurred. In group II recanalization was maintained or improved, in group III vascular occlusion persisted under dalteparin. In group IV we saw complete recanalization in 7/23 (30%), partial recanalization in 7/23 (30%) and no recanalization in 9/23 (40%) cases. The child with PVOD had recanalization proven by lung biopsy; the clinical condition of the child with PPH also improved. Minor bleeding occurred in 2/48 (4%) children. For prophylaxis 95 ± 52 (mean and SD) anti-Xa IU/kg BW, for therapy 129 ± 43 (mean and SD) anti-Xa IU/kg BW proved effective. For both prophylaxis and therapy the required dose per kg BW was inversely related with age (r2 = 0.64, P = 0.017; r2 = 0.13, P = 0.013). Conclusion Dalteparin proved to be an effective and well tolerated drug for prophylaxis and therapy of thrombosis in pediatric patients. Dose requirement for effective treatment was higher in younger children and decreased with age.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/PL00014339
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